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空泡性脊髓病在获得性免疫缺陷综合征患者中,病理表现类似于亚急性联合变性。

Vacuolar myelopathy pathologically resembling subacute combined degeneration in patients with the acquired immunodeficiency syndrome.

作者信息

Petito C K, Navia B A, Cho E S, Jordan B D, George D C, Price R W

出版信息

N Engl J Med. 1985 Apr 4;312(14):874-9. doi: 10.1056/NEJM198504043121402.

DOI:10.1056/NEJM198504043121402
PMID:3974673
Abstract

Twenty of 89 consecutive patients with the acquired immunodeficiency syndrome (AIDS) in whom autopsies were performed over a 3 1/2-year period had a vacuolar myelopathy that was most severe in the lateral and posterior columns of the thoracic cord. Light and electron microscopy showed that vacuoles were surrounded by a thin myelin sheath and appeared to arise from swelling within myelin sheaths. Signs and symptoms referable to the spinal-cord lesions, including paraparesis, often accompanied by spasticity or ataxia (or both), were present in all five patients with marked pathological changes, in five of seven patients with moderate changes, and in two of eight patients with mild changes. Fourteen patients were demented. The clinical presentation was sufficiently distinctive to provide a guide for antemortem diagnosis. Possible causes of the vacuolar changes include uncharacterized viral infection or a metabolic derangement related to selective nutritional deficiency.

摘要

在3年半的时间里,对89例连续的获得性免疫缺陷综合征(AIDS)患者进行了尸检,其中20例患有空泡性脊髓病,这种脊髓病在胸段脊髓的侧柱和后柱最为严重。光镜和电镜检查显示,空泡被一层薄的髓鞘包围,似乎是由髓鞘内肿胀引起的。所有5例有明显病理改变的患者、7例有中度改变的患者中的5例以及8例有轻度改变的患者中的2例,均出现了与脊髓病变相关的体征和症状,包括轻截瘫,常伴有痉挛或共济失调(或两者兼有)。14例患者患有痴呆。临床表现具有足够的特异性,可为生前诊断提供指导。空泡改变的可能原因包括未明确的病毒感染或与选择性营养缺乏相关的代谢紊乱。

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